Medical Therapy. Efficacy or Lack of Efficacy

Prostaglandin-E1 infusion in persistent pulmonary hypertension of the newborn

Stephanie M. Tsoi, Hythem Nawaytou, Hassan Almeneisi, Martina Steurer, Yili Zhao, Jeffrey R. Fineman, Roberta L. KellerUniversity of California San Francisco.United States Pediatric PulmonologyPediatr Pulmonol 2023; DOI: 10.1002/ppul.26759 AbstractBackground: Neonates with persistent pulmonary hypertension of the newborn (PPHN) can present with hypoxia and right ventricular dysfunction with resultant inadequate oxygen delivery and end-organ damage. This study describes […]

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Progression of vascular remodeling in pulmonary vein obstruction

Naoki Masaki, Osamu Adachi, Shintaro Katahira, Yuriko Saiki, Akira Horii, Shunsuke Kawamoto, Yoshikatsu SaikiTohoku University Graduate School of Medicine. Japan Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2020; 160: 777-790.e5DOI: 10.1016/j.jtcvs.2020.01.098 AbstractObjectives: Pulmonary vein obstruction (PVO) frequently occurs after repair of total anomalous pulmonary vein connection with progression of intimal hyperplasia from the anastomotic site

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Outcomes of surgery for young children with multivessel pulmonary vein stenosis

Luis G. Quinonez, Kimberlee Gauvreau, Michele Borisuk, Christina Ireland, Audrey M. Marshall, John E. Mayer, Kathy J. Jenkins, Francis E. Fynn-Thompson, Christopher W. BairdBoston Children’s Hospital.United States Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2015; 150: 911-917DOI: 10.1016/j.jtcvs.2015.06.050 AbstractObjective: We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless

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Selexipag for the Treatment of Pediatric Pulmonary Hypertension: A Systematic Review

Meng Li, Lin Liu, Cong Liu, Zebin Chen, Weibin Li, Xuejuan Li, Xiaopeng Ma, Yumao ZhangShenzhen Children’s Hospital. Eighth Affiliated Hospital and Sun Yat-sen University.China Clinical TherapeuticsClin Ther 2023; DOI: 10.1016/j.clinthera.2023.09.026 AbstractPurpose: To systematically evaluate the safety, dosing regimen, and efficacy of selexipag for pediatric patients with pulmonary hypertension (PH).Methods: A literature search of the electronic databases of

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Treprostinil effectiveness in higher-risk pediatric patients with Idiopathic and Heritable Pulmonary Arterial Hypertension

Yuan He, Qiangqiang Li, Chen Zhang, Bradley B. Keller, Hong GuBeijing Anzhen Hospital and Capital Medical University. Cincinnati Children’s Hospital Medical Center. China and United States Canadian Journal of CardiologyCan J Cardiol 2023; DOI: 10.1016/j.cjca.2023.11.004 AbstractIntroduction: Little is known about the effectiveness of treprostinil in higher-risk pediatric patients with various PAH genotypes. This study was designed to

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Management of systemic to pulmonary shunts and elevated pulmonary vascular resistance

Alexandra N. Linder, Jill Hsia, Sheila V. Krishnan, Emile A. Bacha, Sarah Crook, Erika B. Rosenzweig, Usha S. KrishnanNew York Presbyterian-Morgan Stanley Children’s Hospital and Columbia University Irving Medical Center. Children’s Hospital of Philadelphia. Stony Brook University Children’s Hospital. United States European Respiratory Journal Open ResearchERJ Open Res 2023; 9: DOI: 10.1183/23120541.00271-2023 AbstractBackground: Repair of systemic to

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Pulmonary vasodilators and exercise in Fontan circulation: a systematic review and meta-analysis

Diamantis Kosmidis, Alexandra Arvanitaki, Ioannis T Farmakis, Aris Liakos, Andreas Giannopoulos, Antonios Ziakas, George GiannakoulasAristotle University of Thessaloniki. Royal Brompton Hospital. University Medical Center Mainz. Greece, United Kingdom and Germany HeartHeart 2023; DOI: 10.1136/heartjnl-2023-323166 AbstractObjective: In Fontan circulation, pulmonary arterial hypertension (PAH)-targeted therapies could improve the patients’ exercise capacity. This study aimed to investigate the effects of PAH

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CAR Selectively Enhances the Pulmonary Vasodilatory Effect of Fasudil in a Microsphere Model of Pulmonary Hypertension

Abraham Rothman, Humberto Restrepo, William N. Evans, Valeri Sarukhanov, David MannUniversity of Nevada Las Vegas. Vascular BioSciences.United States Open Respiratory Medicine JournalOpen Respir Med J 2023; DOI: 10.2174/18743064-v17-e230404-2022-19 AbstractBackground: Despite the approval of several medications for pulmonary hypertension, morbidity and mortality are unacceptably high. Systemic hypotension may limit the use of pulmonary hypertension medications.Objectives: This study aimed to

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Kids Mod PAH trial: A multicenter trial comparing mono- versus duo-therapy for initial treatment of pediatric pulmonary hypertension

Joseph M. Collaco, Steven H. Abman, Eric D. Austin, Catherine M. Avitabile, Angela Bates, Jeffrey R. Fineman, Grace A. Freire, Stephanie S. Handler, Dunbar D. Ivy, Usha S. Krishnan, Mary P. Mullen, Nidhy P. Varghese, Delphine Yung, Melanie K. Nies, Allen D. Everett, Kanecia O. Zimmerman, William Simmons, Hrishikesh Chakraborty, Gayane Yenokyan, Allison Newell‐Sturdivant, Eric

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Rapidly Progressive Idiopathic Pulmonary Arterial Hypertension in a Paediatric Patient Treated with Lung Transplantation

Filip Baszkowski, Weronika Pelczar-Płachta, Nikola Pempera, Sylwia Sławek-Szmyt, Marta Kałuzna-Oleksy, Maciej Lesiak, Waldemar BobkowskiPoznan University of Medical Sciences.Poland DiagnosticsDiagnostics 2023; DOI: 10.3390/diagnostics13203185 AbstractPulmonary arterial hypertension (PAH) is a rare heterogeneous disorder in the paediatric population which is mostly associated with congenital heart disease. The management of paediatric idiopathic PAH (IPAH) is difficult due to insufficient comparative

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